Publications by Bernard Schneider

Cerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration

Background: Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) share overlapping clinical, genetic, and neuroimaging features; a spectrum of conditions commonly referred to as the ALS-FTD continuum. The majority of imaging studies focus ...

2025

Optogenetic modulation of peripheral nociceptive neurons with biocompatible optoelectronic implants

Bernard Schneider, Frédéric Pierre Gérald Michoud, Stéphanie Lacour, Ivan Furfaro, Valentina Marie Paggi

Hyperexcitability of peripheral sensory neurons plays a critical role in the development and maintenance of chronic pain. Pharmacological analgesics used in clinics reduce neuronal activity. They often come with non‐negligible side effects. Optogenetic app ...

Wiley2025

Single-cell and spatial atlases of spinal cord injury in the Tabulae Paralytica

Bernard Schneider, Grégoire Courtine, Quentin Barraud, Michael V. Sofroniew, Jocelyne Bloch, Claudia Kathe, Nicholas David James, Matthieu Pierre Gautier, Thomas Haynes Hutson, Michael Alexander Skinnider

Here, we introduce the Tabulae Paralytica—a compilation of four atlases of spinal cord injury (SCI) comprising a single-nucleus transcriptome atlas of half a million cells, a multiome atlas pairing transcriptomic and epigenomic measurements within the same ...

Springer Science and Business Media LLC2024

Liver SMN restoration rescues the Smn2B/- mouse model of spinal muscular atrophy

Bernard Schneider

Background: The liver is a key metabolic organ, acting as a hub to metabolically connect various tissues. Spinal muscular atrophy (SMA) is a neuromuscular disorder whereby patients have an increased susceptibility to developing dyslipidaemia and liver stea ...

2024

Interactions of VMAT2 with CDCrel-1 and Parkin in Methamphetamine Neurotoxicity

Bernard Schneider

In recent years, methamphetamine (METH) misuse in the US has been rapidly increasing, and there is no FDA-approved pharmacotherapy for METH use disorder (MUD). In addition to being dependent on the drug, people with MUD develop a variety of neurological pr ...

2024

Production and Purification of Adeno-Associated Viral Vectors (AAVs) Using Orbitally Shaken HEK293 Cells

Bernard Schneider, Pamela Solange Valdés Undurraga

Humana Press2024

Neurotrauma treatment

Bernard Schneider, Grégoire Courtine, Mark Andrew Anderson, Jordan Squair

The invention provides pharmaceutical compositions, combinations and vectors, such as expression vectors, as well as use thereof in a method for central nervous system repair after neurotrauma, such as spinal cord injury (SCI) or stroke and in a method for ...

2024

TNFα prevents FGF4-mediated rescue of astrocyte dysfunction and reactivity in human ALS models

Bernard Schneider

Astrocytes play a crucial role in the onset and progression of amyotrophic lateral sclerosis (ALS), a fatal disorder marked by the degeneration of motor neurons (MNs) in the central nervous system. Although astrocytes in ALS are known to be toxic to MNs, t ...

Elsevier BV2024

Mitofusin-2 in nucleus accumbens D2-MSNs regulates social dominance and neuronal function

Bernard Schneider, Maria del Carmen Sandi Perez, Olivia Zanoletti, Jocelin Grosse, Elias Georges Gebara, Marie-Isabelle Guillot de Suduiraut, Simone Astori, Sriparna Ghosal, Eva Ramos Fernandez, Alessandro Chioino

The nucleus accumbens (NAc) is a brain hub regulating motivated behaviors, including social competitiveness. Mitochondrial function in the NAc links anxiety with social competitiveness, and the mitochondrial fusion protein mitofusin 2 (Mfn2) in NAc neurons ...

2023

Long term peripheral AAV9-SMN gene therapy promotes survival in a mouse model of spinal muscular atrophy

Bernard Schneider

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by motor neuron loss and skeletal muscle atrophy. SMA is caused by the loss of the SMN1 gene and low SMN protein levels. Current SMA therapies work by increasing SM ...

Oxford University Press2023

Recovery of walking after paralysis by regenerating characterized neurons to their natural target region

Bernard Schneider, Grégoire Courtine, Quentin Barraud, Laetitia Danielle Philippine Baud, Mark Andrew Anderson, Katia Galan, Newton Cho, Jocelyne Bloch, Claudia Kathe, Nicholas David James, Marco Milano, Jordan Squair, Matthieu Pierre Gautier, Anna Lasne, Thomas Haynes Hutson, Steven Lee Ceto, Michael Alexander Skinnider

Axon regeneration can be induced across anatomically complete spinal cord injury (SCI), but robust functional restoration has been elusive. Whether restoring neurological functions requires directed regeneration of axons from specific neuronal subpopulatio ...

American Association for the Advancement of Science2023

Stable isotope labeling and ultra-high-resolution NanoSIMS imaging reveal alpha-synuclein-induced changes in neuronal metabolism in vivo

Bernard Schneider, Graham Knott, Bohumil Maco, Anders Meibom, Stéphane Laurent Escrig, Sofia Carmen Suzanne Spataro, Louise Helene Soegaard Jensen

In Parkinson's disease, pathogenic factors such as the intraneuronal accumulation of the protein alpha-synuclein affect key metabolic processes. New approaches are required to understand how metabolic dysregulations cause degeneration of vulnerable subtype ...

2023

AAV9-mediated SMN gene therapy rescues cardiac desmin but not lamin A/C and elastin dysregulation in Smn(2B/-) spinal muscular atrophy mice

Bernard Schneider

Structural, functional and molecular cardiac defects have been reported in spinal muscular atrophy (SMA) patients and mouse models. Previous quantitative proteomics analyses demonstrated widespread molecular defects in the severe Taiwanese SMA mouse model. ...

Oxford University Press2023

Differential effect of Fas activation on spinal muscular atrophy motoneuron death and induction of axonal growth

Bernard Schneider

Amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA) are the most common motoneuron diseases affecting adults and infants, respectively. ALS and SMA are both characterized by the selective degeneration of motoneurons. Although different in ...

2023

Astrocyte-targeting RNA interference against mutated superoxide dismutase 1 induces motoneuron plasticity and protects fast-fatigable motor units in a mouse model of amyotrophic lateral sclerosis

Patrick Aebischer, Bernard Schneider, Cylia Cloé Rochat, Nathalie Marie Géraldine Bernard

In amyotrophic lateral sclerosis (ALS) caused by SOD1 gene mutations, both cell-autonomous and noncell-autonomous mechanisms lead to the selective degeneration of motoneurons (MN). Here, we evaluate the therapeutic potential of gene therapy targeting mutat ...

WILEY2022

Dopamine and Methamphetamine Differentially Affect Electron Transport Chain Complexes and Parkin in Rat Striatum: New Insight into Methamphetamine Neurotoxicity

Bernard Schneider, Akhil Sharma

Methamphetamine (METH) is a highly abused psychostimulant that is neurotoxic to dopaminergic (DAergic) nerve terminals in the striatum and increases the risk of developing Parkinson's disease (PD). In vivo, METH-mediated DA release, followed by DA-mediated ...

MDPI2022

Gene therapy against amyotrophic lateral sclerosis: a bicistronic AAV vector for RNAi against mutated SOD1

Philippe Colin, Bernard Schneider, Aline Aebi, Pamela Solange Valdés Undurraga, Jean Philippe Gaudry

MARY ANN LIEBERT, INC2022

Central and peripheral delivered AAV9-SMN are both efficient but target different pathomechanisms in a mouse model of spinal muscular atrophy

Bernard Schneider

Spinal muscular atrophy (SMA) is a neuromuscular disease caused by loss of the SMN1 gene and low SMN protein levels. Although lower motor neurons are a primary target, there is evidence that peripheral organ defects contribute to SMA. Current SMA gene ther ...

2022

SMN Depleted Mice Offer a Robust and Rapid Onset Model of Nonalcoholic Fatty Liver Disease

Bernard Schneider

BACKGROUND & AIMS: Nonalcoholic fatty liver disease (NAFLD) is considered a health epidemic with potential devastating effects on the patients and the healthcare systems. Current preclinical models of NAFLD are invariably imperfect and generally take a lon ...

2021

CSP alpha reduces aggregates and rescues striatal dopamine release in alpha-synuclein transgenic mice

Bernard Schneider

alpha-Synuclein aggregation at the synapse is an early event in Parkinson's disease and is associated with impaired striatal synaptic function and dopaminergic neuronal death. The cysteine string protein (CSP alpha) and alpha-synuclein have partially overl ...

2021